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Record Information
Version3.6
Creation Date2006-05-22 15:12:37 UTC
Update Date2016-02-11 01:06:05 UTC
HMDB IDHMDB03157
Secondary Accession NumbersNone
Metabolite Identification
Common NameGuanidinosuccinic acid
DescriptionGuanidinosuccinic acid (GSA) is one of the earliest uremic toxins isolated and its toxicity identified. Its metabolic origins show that it arose from the oxidation of argininosuccinic acid (ASA) by free radicals. The stimulus for this oxidation, occurring optimally in the presence of the failed kidney, is the rising level of urea which, through enzyme inhibition, results in a decline in hepatic levels of the semi-essential amino acid, arginine. It is further noted that concentrations of GSA in both serum and urine decline sharply in animals and humans exposed to the essential amino acid, methionine. Uremic patients suffer from a defective ability to generate methyl groups due to anorexia, dietary restrictions and renal protein leakage. This leads to the accumulation of homocysteine, a substance known to produce vascular damage. Even in healthy subjects intake of choline together with methionine is insufficient to satisfy total metabolic requirements for methyl groups. In end-stage renal disease, therefore, protein restriction contributes to the build-up of toxins in uremia. Replacement using specific amino acid mixtures should be directed toward identified deficiencies and adequacy monitored by following serum levels of the related toxins, in this case GSA and homocysteine. (PMID 12701806 ).
Structure
Thumb
Synonyms
ValueSource
(2S)-2-Carbamimidamidobutanedioic acidChEBI
Guanidinosuccinic acidChEBI
L-N-Amidinoaspartic acidChEBI
N-(Aminoiminomethyl)-L-aspartic acidChEBI
(2S)-2-CarbamimidamidobutanedioateGenerator
(2S)-2-(diaminomethylideneamino)ButanedioateGenerator
GuanidinosuccinateGenerator
L-N-AmidinoaspartateGenerator
N-(Aminoiminomethyl)-L-aspartateGenerator
N-amidino-L-AspartateHMDB
N-amidino-L-Aspartic acidHMDB
N-Carbamimidoyl-L-aspartic acidHMDB
Chemical FormulaC5H9N3O4
Average Molecular Weight175.1427
Monoisotopic Molecular Weight175.059305791
IUPAC Name(2S)-2-carbamimidamidobutanedioic acid
Traditional NameN-amidino-L-aspartic acid
CAS Registry Number6133-30-8
SMILES
NC(=N)N[C@@H](CC(O)=O)C(O)=O
InChI Identifier
InChI=1S/C5H9N3O4/c6-5(7)8-2(4(11)12)1-3(9)10/h2H,1H2,(H,9,10)(H,11,12)(H4,6,7,8)/t2-/m0/s1
InChI KeyInChIKey=VVHOUVWJCQOYGG-REOHCLBHSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as alpha amino acids and derivatives. These are amino acids in which the amino group is attached to the carbon atom immediately adjacent to the carboxylate group (alpha carbon), or a derivative thereof.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentAlpha amino acids and derivatives
Alternative Parents
Substituents
  • Alpha-amino acid or derivatives
  • N-substituted-alpha-amino acid
  • Amino fatty acid
  • Fatty acyl
  • Fatty acid
  • Dicarboxylic acid or derivatives
  • Guanidine
  • Carboximidamide
  • Carboxylic acid
  • Hydrocarbon derivative
  • Organooxygen compound
  • Organonitrogen compound
  • Imine
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
Biofunction
  • Protein synthesis, amino acid biosynthesis
ApplicationNot Available
Cellular locations
  • Cytoplasm (predicted from logP)
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogP-2.068Not Available
Predicted Properties
PropertyValueSource
Water Solubility2.82 mg/mLALOGPS
logP-1.6ALOGPS
logP-3.3ChemAxon
logS-1.8ALOGPS
pKa (Strongest Acidic)2.99ChemAxon
pKa (Strongest Basic)12.23ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count7ChemAxon
Hydrogen Donor Count5ChemAxon
Polar Surface Area136.5 Å2ChemAxon
Rotatable Bond Count4ChemAxon
Refractivity47.25 m3·mol-1ChemAxon
Polarizability15.09 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-004i-1900000000-466615ceb5129f085333View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-00di-9000000000-e5868bbf32c1407fa030View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-006x-9000000000-f34cd801b9c1e714a6fcView in MoNA
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid Locations
  • Blood
  • Cellular Cytoplasm
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue LocationNot Available
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.75 (0.37-1.13) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.259 +/- 0.096 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.4 +/- 0.2 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.17 +/- 0.057 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.4 +/- 0.2 uMAdult (>18 years old)Not SpecifiedNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified0.18 +/- 0.14 uMAdult (>18 years old)BothNormal details
Cellular CytoplasmDetected and Quantified0.2 +/- 0.5 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified0.09 (0.05-0.13) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified<8.6 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified4.0 +/- 2.0 umol/mmol creatinineAdult (>18 years old)BothNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothNormal details
UrineDetected and Quantified1.48 (1.06-1.90) umol/mmol creatinineAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.11 +/- 0.106 uMAdult (>18 years old)BothCirrhosis details
BloodDetected and Quantified20.1 +/- 1.6 uMAdult (>18 years old)BothChronic renal failure details
BloodDetected and Quantified8.17 +/- 1.71 uMAdult (>18 years old)Bothuremia details
Associated Disorders and Diseases
Disease References
Chronic renal failure
  1. Kikuchi T, Orita Y, Ando A, Mikami H, Fujii M, Okada A, Abe H: Liquid-chromatographic determination of guanidino compounds in plasma and erythrocyte of normal persons and uremic patients. Clin Chem. 1981 Nov;27(11):1899-902. [7296840 ]
Cirrhosis
  1. Marescau B, De Deyn PP, Holvoet J, Possemiers I, Nagels G, Saxena V, Mahler C: Guanidino compounds in serum and urine of cirrhotic patients. Metabolism. 1995 May;44(5):584-8. [7752905 ]
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB023116
KNApSAcK IDNot Available
Chemspider ID388951
KEGG Compound IDC03139
BioCyc IDCPD-599
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB03157
Metagene LinkHMDB03157
METLIN ID10
PubChem Compound439918
PDB IDNot Available
ChEBI ID17072
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Cohen BD: Methyl group deficiency and guanidino production in uremia. Mol Cell Biochem. 2003 Feb;244(1-2):31-6. [12701806 ]
  2. Mizutani N, Hayakawa C, Ohya Y, Watanabe K, Watanabe Y, Mori A: Guanidino compounds in hyperargininemia. Tohoku J Exp Med. 1987 Nov;153(3):197-205. [3433275 ]
  3. Torremans A, Marescau B, Van Dam D, Van Ginneken C, Van Meir F, Van Bogaert PP, D'Hooge R, de Vente J, De Deyn PP: GSA: behavioral, histological, electrophysiological and neurochemical effects. Physiol Behav. 2005 Feb 15;84(2):251-64. Epub 2005 Jan 8. [15708777 ]